D-penicillamine, scleroderma

Trade Names

Cuprimine; Depen.

Class and Category

A copper chelating agent that also blocks aldehyde groups involved in intermolecular cross-links in collagen. Early open label trials suggested that D-penicillamine was effective in scleroderma, resulting in improvements in skin thickening and in progression of internal organ involvement. A large, randomized controlled trial of low dose (62.5 mg daily) vs. high dose (750 mg daily) D-penicillamine, however, failed to demonstrate significant differences in skin score, mortality, or incidence of renal crisis.

Indications

Scleroderma.

Dosage

125–250 mg/daily.

Contraindications

Allergy to penicillin.

Adverse Reactions

Common: anorexia, epigastric pain, nausea, vomiting, myelosuppression, rash, taste disturbance.

Serious: aplastic anemia, agranulocytosis, thrombocytopenia, glomerulopathy, renal vasculitis, Goodpasture's syndrome, myasthenia gravis, obliterative bronchiolitis, optic neuritis, peripheral sensory and motor neuropathies, tinnitus,...